儿科ANCA相关的小儿血球炎的临床病理学特征
Olga Charnaya1, Jacqueline Kruglyakova2, Binil Jacob3,4
1Department of Pediatrics, Johns Hopkins School of Medicine, 200 N. Wolfe St., Room 3061, Baltimore, MD, 21287, USA. ocharna1@jhmi.edu.
Pediatric nephrology (Berlin, Germany)
|May 23, 2024
概括
儿科抗中性粒细胞质抗体相关的小儿血球蛋白炎是罕见的和严重的. 淋巴结核硬化,间歇性纤维化和蛋白尿预测患有这种疾病的儿童的功能衰竭或死亡.
科学领域:
- 儿科脏病学 儿科脏病学
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
背景情况:
- 抗中性粒细胞质抗体 (ANCA) 相关的血管炎和血球炎 (AAV) 在儿童中不常见.
- 鉴定组织学和临床特征对于理解儿科AAV至关重要.
- 对这种罕见疾病来说,确定不良结果的风险因素至关重要.
研究的目的:
- 在儿科患者中描述ANCA关联型血球炎 (AAGN) 的组织学和临床特征.
- 在这个队列中,确定与不良结果相关的风险因素,特别是功能衰竭和/或死亡.
- 分析随着时间的推移治疗方式的趋势.
主要方法:
- 在2002年至2022年期间通过脏活检诊断出AAGN的儿科患者 (<20岁) 的回顾性单中心队列研究.
- 从医疗记录中提取组织学和临床特征.
- 对临床,实验室和组织学发现的分析,以确定与功能衰竭 (KF) 和/或死亡的关联.
主要成果:
- 确定了17名儿科患者 (7GPA,10MPA),中位数年龄为15岁.
- 七名患者经历了死亡 (n=1) 或功能衰竭 (n=6) 的复合结果.
- 淋巴结核硬化,间歇性纤维化和管状缩 (IFTA),C3/C1q染色,子内皮质沉积和蛋白尿与终点的危险性增加有关.
结论:
- 儿科AAGN是一种罕见的疾病,具有显著的发病率.
- 淋巴结核硬化和组织学上的IFTA,以及初始蛋白尿,被确定为衰竭或死亡的危险因素.
- 在这个队列中,治疗方式和结果之间没有发现任何关联.
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